PB2218: CHILDHOOD MYELPROLIFERATIVE NEOPLASMS; A SINGLE CENTER EXPERIENCE
Authors: Hasanein Ghali, Salma Al-Hadad, Mazin Al-Jadiry
Journal: HemaSphere (2023)

Topic: 16. Myeloproliferative neoplasms - Clinical Background: Because of the rarity of pediatric MPDs, little is known about the natural history, pathology, and molecular features of these cases. Aims: To study the demographic and clinical features with the outcome of myeloproliferative neoplasms in pediatric patients Methods: A retrospective descriptive study. All consecutive pediatric inpatients newly diagnosed with myeloproliferative neoplasm, were enrolled in this study. Forty-five patients were registered as new cases, treated and followed at the pediatric hematology-oncology unit at Children Welfare Teaching Hospital (CWTH)/ Medical City/ Baghdad. The duration was From January 1st 2010 to December 31st 2019 inclusive (Ten years). Results: The study identified 44 patients met the diagnosis of myeloproliferative disorders and who were 14 years or younger. Regarding diagnosis, twenty-five patients were diagnosed with CML, while twelve patients have JMML. One patient was diagnosed with ET and one patient with MF. For CML patients, 18 patients were diagnosed during chronic phase, 4 patients during accelerated phase and another 3 patients during blast crisis phase. There was an equal distribution of gender in the study group (22 for each). The mean and median age for the study group was 6.7 years and 5.9 years respectively (range 3 months-13.3 years). Regarding the main clinical manifestations, pallor was the major presenting feature in 33 patients (75.0%), this was followed by fever in 28 patients (63.6). Considering the hematological indices of the whole cohort, the mean and median hemoglobin were 8.1 gm/dl and 8.0 gm/dl respectively (range 4.4-15.7 gm/dl), the mean and median WBC counts were 159,800/cmm and 119,000/cmm respectively (range 4,500-500,000/cmm), the mean and median platelets count were 263,700/cmm and 86,000/cmm respectively (range 4,000-2,953,000). Leukocytosis (WBC > 50,000-100,000) was reported in 15 patients, while hyperleukocytosis (WBC >100,000) was reported in 24 patients. Thrombocytosis (platelets > 450,000) was reported in 4 patients, while extreme thrombocytosis (platelets > 1,000,000) was reported in two patients. In correlation between the hematological parameters of the study group along with the diagnoses, the WBC count showed significant high figures in patients with CML (7/25 leukocytosis > 50,000, 18/25 hyperleukocytosis > 100,000) with a significant p value of 0.02. Out of the entire cohort (44 patients); 19 (patients remained alive (43.2%); 18 patients died (40.9%); 3 patients lost to follow-up; 2 patients were referred to a near-home center for Imatinib disposal in accordance to MOH legislations; and 2 patients were discharged against medical advice. Out of the studied population, 18 patients died. The entire cohort had a three-year overall survival of 68.9% and a five-year survival of 54.4%. further analysis of the survival curve has shown that the five-year survival of CML patients was 67%, the three-year survival of JMML was 38%, and the three-year survival of other MPN was 44%. Summary/Conclusion: The study has confirmed the huge lack of cytogenetic and molecular studies required for the diagnosis of myeloproliferative disorders. It has shown that most of the cases were diagnosed by cytomorphology. No patient was found to be asymptomatic; this was notably different from international studies. The lack of index of suspicion and advanced presentation may stand behind this finding. Higher incidence of accelerated and blast phases of CML may unleash the late presentation. Lack of BMT facilities hinder the better outcome of those cases. Keywords: Myeloid